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1.
Artigo em Inglês | MEDLINE | ID: mdl-39326866

RESUMO

MoS2 not only has unique optoelectronic properties realizing photonic and semiconductor applications but also serves as a promising solid lubricant in tribological three-body contacts due to its advantageous friction and wear behavior. Its functionality is defined by elementary processes including strain, oxidation processes, and material mixing. However, these mechanisms were not elucidated for MoS2 having transferred from the MoS2 film synthesized at the main body to a steel counter body during tribological ball-on-disk tests. Using spatially and spectrally high-resolved Raman spectroscopy, we study the compressive and tensile strain within the MoS2 transfer material and analyze the oxidation of molybdenum, sulfur, and iron. In addition, we elaborate on the impact of transition metals modifying the MoS2 films on the strain distribution and oxidation processes. Decreasing intensities of the MoS2 Raman lines are accompanied with enhanced intensities of sulphur and molybdenum oxide Raman signatures which are particularly agglomerated at the edges of the tribological track. The formation of tribochemical oxides, including Mo4O11 in the Magnéli-phase, depends weakly on the type of modifying element, and an oxidation of a modification element itself is not detected. We also identified a tribologically induced formation of disordered few-layer graphene at counter-body surface areas which experienced weak thermo-mechanical tribological load. Our results characterize structural and chemical features of the MoS2 transfer material, thus predicting material failure at the microscopic level.

2.
J Therm Spray Technol ; 31(1-2): 206-216, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-38624750

RESUMO

Grinding wheels are usually manufactured by powder metallurgical processes, i.e., by molding and sintering. Since this requires the production of special molds and the sintering is typically carried out in a continuous furnace, this process is time-consuming and cost-intensive. Therefore, it is only worthwhile for medium and large batches. Another influencing factor of the powder metallurgical process route is the high thermal load during the sintering process. Due to their high thermal sensitivity, superabrasives such as diamond or cubic boron nitride are very difficult to process in this way. In this study, a novel and innovative approach is presented, in which superabrasive grinding wheels are manufactured by thermal spraying. For this purpose, flat samples as well as grinding wheel bodies were coated by low-pressure (LP) cold gas spraying with a blend of a commercial Cu-Al2O3 cold gas spraying powder and nickel-coated diamonds. The coatings were examined metallographically in terms of their composition. A well-embedded superabrasive content of 12 % was achieved. After the spraying process, the grinding wheels were conditioned and tested for the grinding application of cemented carbides and the topographies of both the grinding wheel and the cemented carbide were evaluated. Surface qualities of the ground surface that are comparable to those of other finishing processes were reached. This novel process route offers great flexibility in the combination of binder and hard material as well as a cost-effective single-part and small-batch production.

3.
Acta Biomater ; 9(10): 8611-23, 2013 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-23542554

RESUMO

A cytocompatible and biocompatible, degradable, open-porous, mechanically adaptable metal scaffold made of magnesium alloy W4 melt-extracted short fibres was fabricated by liquid phase sintering. Cylindrical samples (3×5 mm) of sintered W4 short fibres were evaluated under in vitro (L929, HOB, eudiometer, weight loss) and in vivo conditions (rabbits: 6 and 12 weeks). The in vitro corrosion environment (e.g., temperature, flow, composition of corrosion solution, exposure time) significantly influenced the corrosion rates of W4 scaffolds compared with corrosion in vivo. Corrosion rates under cell culture conditions for 72 h varied from 1.05 to 3.43 mm y(-1) depending on the media composition. Corrosion rates measured in eudiometric systems for 24 h were ~24-27 times higher (3.88-4.43 mm y(-1)) than corrosion in vivo after 6 weeks (0.16 mm y(-1)). Moreover, it was found that the cell culture media composition significantly influences the ionic composition of the extract by selectively dissolving ions from W4 samples or their corrosion products. A pilot in vivo study for 6 and 12 weeks demonstrated active bone remodelling, no foreign body reaction and no clinical observation of gas formation during W4 scaffold implantation. Long-term in vivo studies need to be conducted to prove complete degradation of the W4 scaffold and total replacement by the host tissue.


Assuntos
Ligas/farmacologia , Materiais Biocompatíveis/farmacologia , Alicerces Teciduais/química , Animais , Proliferação de Células/efeitos dos fármacos , Sobrevivência Celular/efeitos dos fármacos , Células Cultivadas , Força Compressiva/efeitos dos fármacos , Corrosão , Meios de Cultura/química , Feminino , Humanos , Processamento de Imagem Assistida por Computador , Camundongos , Microscopia Eletrônica de Varredura , Osteogênese/efeitos dos fármacos , Porosidade , Implantação de Prótese , Coelhos
4.
Childs Nerv Syst ; 15(2-3): 128-30, 1999 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-10230669

RESUMO

A 12-month-old infant presented with cerebral seizures and neurological deficits. MRI scan of the brain and angiography showed massive cerebral venous sinus thrombosis complicated by a dural arteriovenous fistula. Subsequent clotting analysis revealed a protein S deficiency. Screening for inherited coagulation inhibitor deficiency is recommended in children with unexplained or atypical thrombotic events.


Assuntos
Fístula Arteriovenosa/complicações , Malformações Arteriovenosas Intracranianas/complicações , Deficiência de Proteína S/complicações , Trombose dos Seios Intracranianos/complicações , Trombose dos Seios Intracranianos/diagnóstico , Fístula Arteriovenosa/patologia , Angiografia Cerebral , Pré-Escolar , Evolução Fatal , Humanos , Lactente , Malformações Arteriovenosas Intracranianas/genética , Malformações Arteriovenosas Intracranianas/patologia , Imageamento por Ressonância Magnética , Masculino , Deficiência de Proteína S/genética , Convulsões/etiologia
5.
Biochem Med Metab Biol ; 41(2): 85-92, 1989 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-2470395

RESUMO

Ca2+ ions were transported into the cell by incubation of the erythrocyte suspension with ionophore A23187, a lipophil electric neutral ion complexing substance. Erythrocyte aggregation could be increased twice, when doubling the intracellular Ca2+-ion concentration. Our measurements lead to the suggestion that an increase of the cytoplasmatic Ca2+ ion changes the physical and/or biochemical properties of the aggregation receptors on the membrane surface, i.e., cell-protein interactions are regulated by alteration of the intracellular Ca2+-ion concentration.


Assuntos
Cálcio/sangue , Eritrócitos/metabolismo , Adolescente , Adulto , Agregação Celular , Citosol/metabolismo , Feminino , Fibrinogênio/sangue , Hematócrito , Humanos , Masculino , Pessoa de Meia-Idade , alfa-Macroglobulinas/análise
6.
Dtsch Med Wochenschr ; 111(18): 698-701, 1986 May 02.
Artigo em Alemão | MEDLINE | ID: mdl-3486106

RESUMO

Exocrine and endocrine pancreatic functions were studied in 30 patients with homozygotic beta-thalassaemia. All were treated with continuous subcutaneous deferoxamine infusions for a mean period of 30 months. Three patients (aged 18-22 years) had insulin-dependent diabetes, two before and one shortly after the onset of deferoxamine administration. There was no improvement during the treatment. An abnormal glucose tolerance test was demonstrated in 14 patients (47%) before and in seven (23%) during deferoxamine infusion. Enzyme activity of alpha-amylase and lipase as an expression of exocrine pancreatic function was normal in all during the observation period. Improvement in endocrine pancreatic function was apparently age-dependent: the younger the patient at the onset of treatment the more likely is normalization of the oral glucose tolerance test.


Assuntos
Glicemia/análise , Desferroxamina/uso terapêutico , Hemossiderose/tratamento farmacológico , Talassemia/complicações , Adolescente , Adulto , Criança , Pré-Escolar , Desferroxamina/administração & dosagem , Complicações do Diabetes , Feminino , Hemossiderose/etiologia , Humanos , Infusões Parenterais/métodos , Injeções Subcutâneas , Lipase/sangue , Masculino , Talassemia/sangue , alfa-Amilases/sangue
7.
Klin Padiatr ; 198(3): 281-4, 1986.
Artigo em Alemão | MEDLINE | ID: mdl-3723992

RESUMO

Immunocompromised children with acute leukemias and solid tumors are at high risk of fatal varicella infection. Reviewing a total of 242 patients at risk we have found that zoster immune plasma from reconvalescent patients (ZIP) and commercially available specific varicella/zoster immune globulin (VZIG) both prevent fatal disease. In addition, Acyclovir was effective against VZV-infections in this group of patients. We summarize our present policy of prophylaxis and treatment of immunocompromised children with neoplastic disease who have been exposed to VZV.


Assuntos
Aciclovir/uso terapêutico , Herpes Zoster/terapia , Imunização Passiva/métodos , Neoplasias/complicações , Doença Aguda , Criança , Pré-Escolar , Doença de Hodgkin/complicações , Humanos , Leucemia/complicações , Prognóstico
8.
Wien Med Wochenschr ; 136 Spec No: 14-6, 1986.
Artigo em Alemão | MEDLINE | ID: mdl-3548086

RESUMO

Impairment of red cell deformability (and decrease of survival time) is the common trait of congenital haemolytic anemias with the exception of the enzyme defect glucose-6-phosphate-dehydrogenase (GPDH) deficiency. Causes for increased red cell rigidity may be: spherocytosis (familial hemolytic anemia), instable less fluid Hb (instable hemoglobinopathy), abnormal HbS-formation (sickle cell disease), genetic synthesis defect of Hb chains (homozygous beta-thalassemia), enzyme defects (autosomal recessive pyruvate-kinase deficiency). With GPDH red cell deformability remains unchanged. Splenectomy may be beneficial in anemias with erythrocyte rigidification if clinical condition so requires (repeated transfusions, aplastic and hemolytic crises). Assessment of red cell deformability in vitro saves laborous and exposure to radiation involving testing of cell survival time (e.g. radiochrome test).


Assuntos
Anemia Hemolítica Congênita/sangue , Deformação Eritrocítica , Deficiência de Glucosefosfato Desidrogenase/sangue , Anemia Falciforme/sangue , Aberrações Cromossômicas/sangue , Transtornos Cromossômicos , Hemoglobinopatias/sangue , Humanos , Piruvato Quinase/deficiência , Esferocitose Hereditária/sangue , Talassemia/sangue
9.
Dtsch Med Wochenschr ; 111(5): 176-81, 1986 Jan 31.
Artigo em Alemão | MEDLINE | ID: mdl-3943469

RESUMO

Subcutaneous continuous infusions of deferoxamine were administered to 28 children with thalassaemia major. Longitudinal growth beyond the eleventh year was favourably influenced. The normal growth spurt during puberty was not demonstrable in six patients (three boys and three girls) during their puberty. Taking into account standard height allowing for parents' height, prospective final height and ethnic origin, the treatment favourably influenced longitudinal growth in 26 of the 28 children.


Assuntos
Estatura , Desferroxamina/uso terapêutico , Talassemia/tratamento farmacológico , Determinação da Idade pelo Esqueleto , Criança , Pré-Escolar , Desferroxamina/administração & dosagem , Feminino , Grécia/etnologia , Homozigoto , Humanos , Injeções Subcutâneas , Itália/etnologia , Masculino , Talassemia/genética , Talassemia/fisiopatologia , Turquia/etnologia
10.
Monatsschr Kinderheilkd ; 133(12): 876-8, 1985 Dec.
Artigo em Alemão | MEDLINE | ID: mdl-4088285

RESUMO

Antibodies against Yersinia were found in 12 of 50 patients with hemosiderosis, in 11 of 47 patients with thalassemia major and in one of three patients with Blackfan-Diamond anemia. All patients were treated with subcutaneous continuous deferoxamin-infusions. A systemic yersiniosis occurred in seven patients, all with homozygous beta-thalassemia, in five during and in two before treatment with deferoxamin. Hemosiderosis and infusions with deferoxamin seem to increase the risk of yersinia septicemia.


Assuntos
Anticorpos Antibacterianos/análise , Hemossiderose/imunologia , Yersiniose/imunologia , Adolescente , Adulto , Criança , Pré-Escolar , Ferritinas/sangue , Humanos , Ferro/sangue , Esplenectomia , Talassemia/imunologia , Yersinia enterocolitica/imunologia
11.
Dtsch Med Wochenschr ; 110(48): 1848-51, 1985 Nov 29.
Artigo em Alemão | MEDLINE | ID: mdl-4064938

RESUMO

Liver function during continuous subcutaneous deferoxamine therapy was investigated in 29 patients with homozygotic beta-thalassaemia. Average duration of treatment was 26 months (range 8-51 months). A decrease in haemosiderosis and an improvement in liver function was observed in 27 patients: Mean liver density, determined by computed tomography, decreased from 98 to 84 HU, mean serum ferritin concentration fell from 8028 to 3661 ng/ml, mean serum GOT activity from 44 to 13 U/l and GPT from 51 to 16 U/l. Mean cholinesterase activity, reflecting the improved synthetic activity of the liver, increased from 4063 to 4530 U/l.


Assuntos
Desferroxamina/administração & dosagem , Hemossiderose/tratamento farmacológico , Fígado/fisiopatologia , Talassemia/tratamento farmacológico , Adolescente , Adulto , Criança , Pré-Escolar , Hemossiderose/etiologia , Humanos , Injeções Subcutâneas , Testes de Função Hepática , Talassemia/complicações , Talassemia/fisiopatologia , Fatores de Tempo
12.
Klin Wochenschr ; 62(23): 1136-9, 1984 Dec 03.
Artigo em Inglês | MEDLINE | ID: mdl-6521362

RESUMO

In vitro measurements were carried out to study the aggregation of erythrocytes in 33 children and young adolescents, three older adolescents, and 38 adults with type I diabetes. The aggregate formation of erythrocytes in stasis was increased in adult patients with both "good" and "poor" metabolic control when compared to control values. The aggregation of red cells in all children and the younger adolescents, both those under good and poor metabolic control, did not statistically differ from those of controls. Each of the three older adolescents showed an increased aggregate formation of their erythrocytes compared to controls. All adult patients under poor metabolic control exhibited various stages of diabetic retinopathy as shown by fluorescence angiography. Only one child under poor metabolic control showed an early stage of retinopathy (stage I according to Malone). The three older adolescents showed an increased aggregate formation of their erythrocytes. These patients exhibited also stage I of retinopathy. We suggest that the increased aggregation of erythrocytes of both adult and older adolescent patients may be one of the reasons for the development of diabetic retinopathy. Moreover, the normal erythrocyte aggregation of the diabetic children and younger adolescents could help to explain the rare occurrence of microangiopathies in childhood diabetes.


Assuntos
Diabetes Mellitus Tipo 1/sangue , Retinopatia Diabética/etiologia , Agregação Eritrocítica , Adolescente , Adulto , Viscosidade Sanguínea , Criança , Ácidos Difosfoglicéricos/sangue , Deformação Eritrocítica , Humanos
13.
Dtsch Med Wochenschr ; 109(44): 1682-5, 1984 Nov 02.
Artigo em Alemão | MEDLINE | ID: mdl-6489181

RESUMO

Faecal iron excretion during continuous subcutaneous deferoxamine infusion was measured by flameless atom-absorption spectral photometry in 21 patients with homozygous ss-thalassaemia and one patient with Blackfan-Diamond anaemia. Taking into account faecal and urinary iron excretion, more iron was eliminated than was taken up during transfusion and by intestinal absorption. Urine contained 37.9%, faeces 62.1% of the amount of iron eliminated.


Assuntos
Desferroxamina/uso terapêutico , Fezes/análise , Hemossiderose/tratamento farmacológico , Ferro/análise , Adolescente , Criança , Pré-Escolar , Desferroxamina/administração & dosagem , Hemossiderose/etiologia , Hemossiderose/metabolismo , Humanos , Injeções Subcutâneas/métodos , Masculino , Autoadministração , Talassemia/complicações
14.
Klin Padiatr ; 196(3): 162-5, 1984.
Artigo em Alemão | MEDLINE | ID: mdl-6088889

RESUMO

One hundred and twenty-one febrile episodes (FE) were studied in 58 aplastic children with acute leukemia. All patients received a prophylactic regimen of trimethoprim-sulfamethoxazole (TS) and colistin sulfate orally, and amphotericin B locally from the beginning of their antineoplastic therapy. Eighty episodes were analysed retrospectively. Forty-one episodes in 24 patients were treated prospectively with a standard regimen of gentamicin + cefotaxime. The results show that the prophylactic regimen does not eradicate potentially pathogenic organisms from the throat, urine, or stools. Sixty-three per cent of FE were of unknown origin (FUU). 40.5% of all isolated organisms were TS resistant. In the prospective group, 9 of 41 FE (22%) responded to gentamicin + cefotaxime with lysis of fever within 48 hours, and 63% required no further antibiotics. Defeverescence occurred in the whole group within 5 +/- 4 days. No patients were lost to infectious complications. The combination of gentamicin + cefotaxime as initial therapy of a febrile episode must be supplemented within 48 to 72 hours by additional antibiotics in the absence of clinical improvement.


Assuntos
Anfotericina B/uso terapêutico , Infecções Bacterianas/prevenção & controle , Candidíase/prevenção & controle , Colistina/uso terapêutico , Leucemia Linfoide/complicações , Leucemia Mieloide Aguda/complicações , Sulfametoxazol/uso terapêutico , Trimetoprima/uso terapêutico , Anemia Aplástica/complicações , Cefotaxima/uso terapêutico , Criança , Combinação de Medicamentos/uso terapêutico , Quimioterapia Combinada , Febre/tratamento farmacológico , Gentamicinas/uso terapêutico , Humanos , Combinação Trimetoprima e Sulfametoxazol
16.
Blut ; 48(3): 123-9, 1984 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-6697002

RESUMO

A new PK variant with moderate hemolytic anemia is described. The enzymes of the nonanemic parents show sigmoidal reaction kinetics, with normal kinetic parameters, but differ with respect to nucleotide specificity, thermostability, and the concentrations of the glycolytic intermediates in the erythrocytes. The most characteristic features of the patient's (daughter) enzyme are a 30% activity, hyperbolic reaction kinetics and only two bands in the SDS-gel electrophoresis instead of three bands observed with the parental enzymes. Moreover, the pH-optimum is shifted to the acidic range, the affinity for PEP and ADP is decreased, ATP inhibition is negligible and FDP-activation is roughly ten times smaller than with controls. The concentrations of 2,3-DPG, 2-PG and PEP in the erythrocyte are increased, but ATP decreased. As there is no consanguinity in the parents and their enzymes are different this PK mutant can be considered to be compound-heterozygous for two different mutant PK alleles.


Assuntos
Eritrócitos/enzimologia , Variação Genética , Piruvato Quinase/deficiência , Trifosfato de Adenosina/sangue , Adulto , Anemia Aplástica/sangue , Ácidos Difosfoglicéricos/sangue , Eletroforese em Gel de Poliacrilamida , Feminino , Glicerofosfatos/sangue , Humanos , Concentração de Íons de Hidrogênio , Focalização Isoelétrica , Fosfoenolpiruvato/sangue , Piruvato Quinase/sangue , Piruvato Quinase/genética , Dodecilsulfato de Sódio
17.
J Biomech ; 17(4): 263-79, 1984.
Artigo em Inglês | MEDLINE | ID: mdl-6736063

RESUMO

Wall shear distributions during the cardiac cycle at the valve rings of Starr-Edwards, Björk-Shiley and Lillehei-Kaster aortic valves are measured and compared with thresholds reported for shear-induced trauma of blood components. Further, for the disk valves, the influence of pulse rate on wall shear stresses is evaluated. Hot film anemometry with flush-mounted wall shear probes is used as measurement technique in a pulsatile flow mock circuit. The experimental systolic data support the better hemodynamic characteristics of the disk valves over the ball valve also with respect to the threshold shear stresses of flow induced blood trauma. These results are confirmed by postoperative clinical studies, where lower LDH-values are found with the disk than with the ball valves. During diastole, however, high shear stresses are measured and calculated at the valve ring of the Björk-Shiley prosthesis, which can be referred to the non-overlapping closing mechanism. This result is discussed with respect to the often observed thrombus formation at the disk downstream of the smaller orifice of the Björk-Shiley valve.


Assuntos
Próteses Valvulares Cardíacas , Hemodinâmica , Reologia , Estresse Mecânico , Valva Aórtica , Velocidade do Fluxo Sanguíneo , Plaquetas/fisiologia , Eritrócitos/fisiologia , Humanos , Desenho de Prótese , Pulso Arterial
18.
Monatsschr Kinderheilkd ; 131(12): 848-52, 1983 Dec.
Artigo em Alemão | MEDLINE | ID: mdl-6664357

RESUMO

The antibody response to a new, 17-valent pneumococcal vaccine (Moniarix; Smith Kline-RIT) was tested in 43 splenectomised or functionally asplenic patients. The vaccine contained the following types of capsular antigens: 1, 2, 3, 4, 6A, 7F, 8, 9N, 11A, 12F, 14, 15F, 17F, 18C, 19F, 23F, 25 (Danish nomenclature). The vaccination was well tolerated. Local, reversible reddening and swelling was seen in 22 patients. The only systemic side effect was a rise of temperature in 13 patients. Serum titers of more than 300 ng/ml of specific antibodies were considered protective. A complete immunization of all patients was reached only with the capsular antigens 11A and 19F. The other antigens only induced protective serum concentrations in some of the patients. Considering the frequency of infections with the different types of antigens in Western Europe, percentage of asplenic patients at risk for infections with all pneumococcal types has been reduced by the vaccination to about 34%. It is proposed that the combination of pneumococcal vaccine and penicillin-prophylaxis provide effective protection for splenectomised patients against bacterial infections. The duration of penicillin-prophylaxis depends on the age of the patient at the time of splenectomy and the basic disease.


Assuntos
Infecções Bacterianas/prevenção & controle , Vacinas Bacterianas/uso terapêutico , Esplenectomia , Adolescente , Fatores Etários , Criança , Pré-Escolar , Humanos , Penicilinas/administração & dosagem , Penicilinas/uso terapêutico , Complicações Pós-Operatórias , Sepse/prevenção & controle , Streptococcus pneumoniae/imunologia , Fatores de Tempo
19.
Hoppe Seylers Z Physiol Chem ; 364(7): 787-92, 1983 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-6618441

RESUMO

The influence of temperature, K+, Mg2+ and fructose 1,6-bisphosphate on human red cell pyruvate kinase was investigated. Kinetic measurements between 4 degrees C and 43 degrees C revealed a remarkable influence of the temperature on the allosteric behaviour of the enzyme. Below a transition region between 15 degrees C and 20 degrees C (as obtained from an Arrhenius plot) the enzyme shows non-cooperative behaviour, as can be deduced from Michaelis-Menten, Hill and Scatchard plots. At temperatures above 20 degrees C cooperativity increases with rising temperature. This effect becomes even more pronounced at higher temperatures upon addition of increasing amounts of K+ and Mg2+ accompanied by a slight decrease of the reaction velocity. Fructose 1,6-bisphosphate, however, abolishes cooperativity at every temperature and salt concentration measured. Difficulties which arise in evaluating the correct values of V, Km and the Hill coefficient nH with cooperative systems are met by using a computer program of Wieker, Johannes and Hess, especially designed for the determination of kinetic parameters obtained from sigmoidal steady-state kinetics.


Assuntos
Eritrócitos/enzimologia , Piruvato Quinase/sangue , Humanos , Cinética , Magnésio/farmacologia , Potássio/farmacologia , Conformação Proteica , Temperatura
20.
Enzyme ; 29(3): 189-97, 1983.
Artigo em Inglês | MEDLINE | ID: mdl-6851994

RESUMO

Investigations on the activity and the kinetic behavior of red cell pyruvate kinase in the erythrocyte membrane and hemolysate of 6 patients with pyruvate kinase (PK) deficiency are presented and compared with those of normal individuals. Hemolysate: The PK in the hemolysate of normal individuals showed the well-known allosteric properties, reflected by sigmoidal reaction kinetics (Hill-coefficient greater than 1). In contrast to these findings the enzymes of 4 of the patients which had not been splenectomised and which were all in satisfactory condition showed between 15 and 35% of the PK activity of normal individuals in the hemolysates, whereas in the case of the 2 splenectomised patients only 5 and 15% of this activity could be measured. All of the patients' enzymes showed non allosteric (hyperbolic) reaction kinetics (Hill-coefficient approximately 1.0). Red cell membranes: Our studies confirmed earlier reports that not only the hemolysate of the patients, but also their red cell membranes ('ghosts') exhibit PK activity. In normal individuals the activity of the membrane-localised PK is about 5% of the cytoplasmatic one. In case of the membrane-localised enzyme of all patients--irrespective if they were splenectomised or not--the activity is decreased to 30-50%, compared to the activity of ghosts of the control persons. Reaction kinetic studies on the PK isolated from red cell membranes from normal individuals as well as from all 6 patients exhibited hyperbolic Michaelis-Menten kinetics with Hill coefficients around 1.0.


Assuntos
Eritrócitos/enzimologia , Piruvato Quinase/deficiência , Adolescente , Adulto , Citoplasma/enzimologia , Membrana Eritrocítica/enzimologia , Feminino , Humanos , Cinética , Masculino , Piruvato Quinase/sangue
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